A5031693170- Lysosomal Storage Disorders Research
- Glycogen Storage Diseases and Myoclonus
- Autophagy in Disease and Therapy
- Advanced Biosensing Techniques and Applications
- Hereditary Neurological Disorders
- Nanoparticle-Based Drug Delivery
- Muscle Physiology and Disorders
- Neurological diseases and metabolism
- Telomeres, Telomerase, and Senescence
- Biochemical Acid Research Studies
- Neurogenetic and Muscular Disorders Research
- GDF15 and Related Biomarkers
- Nanoplatforms for cancer theranostics
- Biochemical and Molecular Research
- Trypanosoma species research and implications
- bioluminescence and chemiluminescence research
- Mitochondrial Function and Pathology
- Electrochemical sensors and biosensors
- Extracellular vesicles in disease
- Monoclonal and Polyclonal Antibodies Research
- RNA regulation and disease
- Historical and Literary Studies
- Enzyme Structure and Function
- Photoacoustic and Ultrasonic Imaging
- Tissue Engineering and Regenerative Medicine
Oniris
2016-2021
Institut National de Recherche pour l'Agriculture, l'Alimentation et l'Environnement
2017-2019
PAnTher - Physiopathologie Animale et bioThérapie du muscle et du système nerveux
2017-2019
Nerve Centre
2017-2019
Université Bretagne Loire
2017-2018
Université Nantes Angers Le Mans
2016
In order to assess the therapeutic potential of cell-based strategies, it is paramount importance elaborate and validate tools for monitoring behavior injected cells in terms tissue dissemination engraftment properties. Here, we apply bismuth ferrite harmonic nanoparticles (BFO HNPs) vitro expanded human skeletal muscle-derived stem (hMuStem cells), an attractive avenue patients suffering from Duchenne muscular dystrophy (DMD). We demonstrate possibility cell labeling with HNPs. also show...
Pompe disease is a lysosomal storage disorder caused by acid-α-glucosidase (GAA) deficiency, leading to glycogen storage. The manifests as fatal cardiomyopathy in infantile form. Enzyme replacement therapy (ERT) has recently prolonged the lifespan of these patients, revealing new natural history. neurologic phenotype and persistence selective muscular weakness some patients could be attributed central nervous system (CNS) uncorrected ERT. GAA-KO 6neo/6neo mice were treated with single...
Pompe disease, which is due to acid alpha-glucosidase deficiency, characterized by skeletal muscle dysfunction attributed the accumulation of glycogen-filled lysosomes and autophagic buildup. Despite extensive tissue damages, a failure satellite cell (SC) activation lack regeneration have been reported in patients. However, origin this defective program unknown. Additionally, whether these deficits occur gradually over disease course unclear. Using longitudinal pathophysiological study two...
Pompe disease (glycogen storage type II) is a lysosomal disorder due to mutation in the gene that encodes acid alpha-glucosidase (GAA). GAA deficiency causes excessive of glycogen many cell types, leading and, subsequently, tissue dysfunction. Cardiac, respiratory and skeletal muscles are most severely affected. Enzyme replacement therapy (ERT) with recombinant human (rhGAA, Myozyme®, Genzyme, Cambridge) only approved treatment for disease. A new therapeutic strategy was developed consisting...